A 5-month-old boy patient was referred to us because of skin problems. The parents were second cousins. There was no family history of congenital skin or vascular diseases. He was born premature and suffered from hypoxic ischemic encephalopathy, respiratory distress syndrome and septic shock. He was kept in the neonatal intensive care unit for fifty days and had required six cutdown procedures because of management of life-threatening episodes of septic shock. At the age of two months, he developed multiple reticuled, blue-violet skin lesions, midline nevus flammeus along the upper lip, cavernous hemangioma at the frontoparietal region and the chin (Fig. 1.). The skin lesions were distributed all over the body especially below umbilicus. We thought that these features of cutis marmorata telengiectatica congenital (CMTC) might be connected with the vascular trauma because of repeated cutdown procedures, although Doppler study of venous and arterial system was normal. Brain computerized tomography revealed left hemispheric atrophy, asymmetric dilatation of left lateral ventricle and calcification at left basal ganglion level.

Fig. 1. Typical reticulated blue-violet net-work is evident on the left arm, right and left leg (thick arrows). Note nevus flammeus of philtrum and upper lip (dotted arrow) and three cutdown scars (thin arrows).

A number of hypothesis have been proposed for the pathogenesis of CMTC, including environmental factors, autosomal dominant inheritance with low or variable penetrance, a multifactorial cause or a lethal gene surviving mosaism, a peripheral neural dysfunction, and a failure of development of the mesodermic vessels in the early embryonic stage(1-4). We suggest that a failure of development of vessels might be because of septic shock and frequently repeated cut- down procedures. One of the most serious complications of cutdown procedures is probably arterial thrombosis. And also, repeated microthrombosis might lead to increased peripheral flow resistance, which causes a perfusion deficit, and thus explain the reduced oxygen saturation in the tissues and specific skin problems. Although, we could not confirm this theory, we presume a functional malformation at the level of the terminal blood vessels.

Osman Baspinar,
Selim Kervancioglu,*
Departments of Pediatrics,
and *Radiodiagnostics,
Gaziantep University, Faculty of Medicine,
Gaziantep, Turkey.
E-mail: [email protected]